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Update on ARVD and its Look-alikes Stefan L. Zimmerman, MD Assistant Professor of Radiology, Johns Hopkins University School of Medicine Disclosures • None Outline • Background on ARVD • How is ARVD diagnosed • Characteristic CMR findings • Pitfalls and mimics Arrhythmogenic Right Ventricular What is ARVD? Cardiomyopathy/Dysplasia • Rare inherited genetic disorder; defects in the desmosome • Fibrofatty replacement of the right ventricular myocardium • Electrical and structural abnormalities of the right ventricle • Sudden cardiac death Saffitz JE. Annu Rev Path Mech Dis 2011.6:299 Task Force Criteria Circulation 2010;121:1533 • Structure – Imaging – Biopsy • Electrical – EKG, Holter • Family History • Genetics Radiology 2004;232:38-48 MRI Task Force Criteria MAJOR MINOR MRI Task Force Criteria QUALITATIVE AND QUANTITATIVE Qualitative RV findings 24M Marine who presented with sustained ventricular tachycardia (HR Normal RV 230) after a workout 23F with sudden cardiac arrest, positive for two ARVD-causing mutations (DSP, PKP2)

Description:

Structure. – Imaging. – Biopsy. • Electrical. – EKG, Holter. • Family History Qualitative CMR abnormalities –Free wall bulging (dyskinesia).

Update of ARVD and its Look-alikes PDF

45 Pages·2015·2.89 MB·English

by Stefan Zimmerman

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Update on ARVD and its Look-alikes Stefan L. Zimmerman, MD Assistant Professor of Radiology, Johns Hopkins University School of Medicine Disclosures • None Outline • Background on ARVD • How is ARVD diagnosed • Characteristic CMR findings • Pitfalls and mimics Arrhythmogenic Right Ventricular What is ARVD? Cardiomyopathy/Dysplasia • Rare inherited genetic disorder; defects in the desmosome • Fibrofatty replacement of the right ventricular myocardium • Electrical and structural abnormalities of the right ventricle • Sudden cardiac death Saffitz JE. Annu Rev Path Mech Dis 2011.6:299 Task Force Criteria Circulation 2010;121:1533 • Structure – Imaging – Biopsy • Electrical – EKG, Holter • Family History • Genetics Radiology 2004;232:38-48 MRI Task Force Criteria MAJOR MINOR MRI Task Force Criteria QUALITATIVE AND QUANTITATIVE Qualitative RV findings 24M Marine who presented with sustained ventricular tachycardia (HR Normal RV 230) after a workout 23F with sudden cardiac arrest, positive for two ARVD-causing mutations (DSP, PKP2)

Description:

Structure. – Imaging. – Biopsy. • Electrical. – EKG, Holter. • Family History Qualitative CMR abnormalities –Free wall bulging (dyskinesia).

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